Rituximab in the treatment of acquired hemophilia.

OBJECTIVE: To review published literature evaluating the use of rituximab for treatment of acquired hemophilia.

DATA SOURCES: An English-language literature search was conducted using MEDLINE (1966-January 2006). References of identified articles were subsequently reviewed for additional data. Search terms included rituximab, acquired hemophilia, and inhibitors.

DATA SYNTHESIS: Available data suggest that B-cell depletion by rituximab in patients with acquired hemophilia may contribute to the eradication of inhibitors to coagulation factors. B-cells have been shown to be essential in the development of autoimmunity or an acquired immune response. Beneficial effects of rituximab, after failure of established therapies, have been reported in case reports and one small, open-label trial.

CONCLUSIONS: Although data are limited, administration of rituximab appears to be an effective option for treatment of patients with acquired hemophilia after established therapies have failed. Patients given rituximab experienced cessation of recurrent bleeding, normalization of factor VIII activity, and eradication of inhibitors. A complete response to rituximab (undetectable inhibitors, normalization of factor levels) generally occurred several weeks to months after initiation of therapy.